TY - JOUR
T1 - Association between neutropenia and IgG antineutrophil antibodies in a case of CD40LG deficiency due to two novel mutations
AU - Assing, Kristian
AU - Nielsen, Kaspar René
AU - Tenstad, Helene Broch
AU - Jakobsen, Marianne Antonius
AU - Nielsen, Christian
AU - Grosen, Dorthe
AU - Hartling, Ulla Birgitte
PY - 2020/2
Y1 - 2020/2
N2 - This case suggests a mechanistic rationale for the clinical efficacy of intravenous immunoglobulins (IVIG) in treating CD40 ligand (CD40L) deficiency associated neutropenia as it is the first reported instance of free and cell-bound antineutrophil antibodies in a case of CD40L deficiency, accompanied by a prolonged and clinically severe neutropenia.
AB - This case suggests a mechanistic rationale for the clinical efficacy of intravenous immunoglobulins (IVIG) in treating CD40 ligand (CD40L) deficiency associated neutropenia as it is the first reported instance of free and cell-bound antineutrophil antibodies in a case of CD40L deficiency, accompanied by a prolonged and clinically severe neutropenia.
KW - CD40 ligand deficiency
KW - hyper-IgM syndrome
KW - neutropenia
KW - neutrophil IgG auto-antibodies
U2 - 10.1002/ccr3.2621
DO - 10.1002/ccr3.2621
M3 - Journal article
C2 - 32128179
AN - SCOPUS:85077159415
SN - 2050-0904
VL - 8
SP - 313
EP - 316
JO - Clinical Case Reports
JF - Clinical Case Reports
IS - 2
ER -